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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 31  |  Issue : 3  |  Page : 205-207

Multilocular cystic renal cell carcinoma: A rare case report with review of literature


Department of Pathology, Dr. D. Y. Patil Medical College, Pune, Maharashtra, India

Date of Web Publication17-Aug-2017

Correspondence Address:
Shirish S Chandanwale
75/1+2/1, Krishna Appt., New Sangvi Pune - 411 027, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jms.jms_66_16

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  Abstract 


Multilocular cystic renal cell carcinoma (MCRCC) is a rare adult cystic tumor of the kidney and is considered as a distinct type of clear cell renal cell carcinoma. Most patients are asymptomatic and they are detected incidentally. MCRCC is consistently low nuclear grade and has an excellent prognosis. Concern still persists regarding its true malignant potential. Although surgical resection is curative, lymph node metastasis has been reported recently. There are very few reports on large series of cases and limited available follow-up. We report a case of MCRCC in a 60-year-old male patient who presented with abdominal distention. Careful follow-up is essential in these patients for better prognosis.

Keywords: Clear, multilocular, renal cell carcinoma


How to cite this article:
Chandanwale SS, Jadhav RS, Rao R, Buch A, Nikam S. Multilocular cystic renal cell carcinoma: A rare case report with review of literature. J Med Soc 2017;31:205-7

How to cite this URL:
Chandanwale SS, Jadhav RS, Rao R, Buch A, Nikam S. Multilocular cystic renal cell carcinoma: A rare case report with review of literature. J Med Soc [serial online] 2017 [cited 2019 Jul 17];31:205-7. Available from: http://www.jmedsoc.org/text.asp?2017/31/3/205/213112




  Introduction Top


Multilocular cystic renal cell carcinoma (MCRCC) is a rare cystic tumor of the kidney. It is considered as a distinct type of clear cell renal cell carcinoma (RCC) in the 2004 WHO classification.[1],[2] Most patients are asymptomatic and they are detected incidentally. MCRCC is consistently low nuclear grade and has an excellent prognosis. Surgical resection is curative.[3] Some concerns regarding its malignant potential and clinical significance still persist because there are very few follow up studies on large series of cases. We report a case of MCRCC in a 60-year-old male patient.


  Case Report Top


A 60-year-old male presented with mass and pain in the left abdomen for 15 days. He had a history of intermittent fever and incomplete defecation. There was no other significant medical history. Ultrasonography showed 75 cm × 70 cm well-defined heterogeneous mass in the left lower pole kidney with marked cystic change. There was increased central and peripheral vascularity. Contrast-enhanced computed tomography showed well-defined thick-walled cystic lesion. RCC with cystic change was suspected. The patient underwent left nephrectomy and specimen was received for histopathological examination.

Gross examination of the kidney showed a tumor mass in the lower pole of the kidney. Cut surface showed well-encapsulated multicystic tumor mass measuring 7 cm × 6.5 cm. Thin and thick septa were seen in between the cysts and they were filled with gelatinous brown-colored fluid [Figure 1]. There were no para-aortic lymph nodes.
Figure 1: Cystic mass in the lower pole of the kidney

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Histopathological examination of tumor showed multiple cysts of varying sizes filled with eosinophilic fluid and hemorrhage [Figure 2]a. Cyst wall had thin fibrous septa and was lined by one to three layers of neoplastic epithelial cells. Tumor cells had well-defined cytoplasmic borders, abundant clear cytoplasm, and small round hyperchromatic nuclei with inconspicuous nucleoli [Figure 2]b. Stroma was scanty, hypocellular, and made up of benign spindle cells. Few small groups of similar looking tumor cells were seen in stroma [Figure 2]c. Lymphatic and vascular invasion was not seen. Capsule was free of tumor cells. Tumor cells showed strong immunoreactivity with epithelial membrane antigen [[Figure 2]d, arrows]. A histopathological diagnosis of MCRCC was made.
Figure 2: (a) Tumor shows multiple cysts filled with eosinophilic fluid (H and E, ×100). (b) Cysts lined by tumor cells with abundant clear cytoplasm and small round hyperchromatic nuclei with inconspicuous nucleoli (H and E, ×400). (c) Small groups of tumor cells in stoma (H and E, ×100). (d) Epithelial membrane antigen-positive tumor cells (immunohistochemistry, ×100)

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  Discussion Top


RCC accounts for 80%–85% of primary malignant neoplasms of the kidney.[3] MCRCC accounts for 1%–2% of all renal tumors.[1] They are found in the age group of 20–77 years with mean age of 51 years. Tumors have male preponderance with male to female ratio of 3:1.

Due to few reports on large series of cases and limited available follow-up, the diagnosis of MCRCC is based on more restrictive 2004 WHO criteria.[4],[5]

MCRCC should be used exclusively to identify those cystic RCC with small volume (25% or less) of neoplastic clear cells in the cyst walls. Tumors are usually of low Fuhrman nuclear grade (1 or 2) and usually confined to the kidney. Gross features include multilocular cystic appearance, variegated yellowish solid component limited to small areas with no expansive nodules or necrosis.[1],[4],[5]

Most patients are asymptomatic and MCRCC is incidentally detected.[3] In our case, the patient complained of palpable mass in the left lower abdomen. Tumor in our case was multicystic and showed fibrous septa. There were no expansile solid nodules or areas of necrosis grossly. Tumor appeared to be well encapsulated (pseudocapsule) at places. Histological features observed in our case were pseudocapsule and multilocular cyst lined by fibrous septa and filled with gelatinous fluid with scanty hypocellular fibrous stroma. Septa were lined by one or more layers of neoplastic epithelial cells with abundant clear cytoplasm and hyperchromatic nuclei with inconspicuous nucleoli. Nuclei were of Fuhrman Grade 1. Necrosis was not seen. Occasional papillary tufting in the cyst wall, foci of calcification, and osseous metaplasia are seen in MCRCC. These features were not seen in our case.

The important differential diagnosis of MCRCC in adults includes benign multilocular cyst of the kidney, cystic nephroma, cystic clear cell RCC, and tubulocystic carcinoma.[5]

Limited available follow-up and difficulties in the differential diagnosis are responsible for the variable outcome observed in some series in which cystic RCCs are incorporated into the analysis in addition to true MCRCC; therefore, some controversy exists in the literature regarding its malignant potential.[1],[3],[5]

The absence of hobnail quality of lining epithelium of the cyst, ovarian-like stroma, papillary structure, expansile nodules of clear cells, tubular structures, nuclear atypia, and prominent nucleoli ruled out the differential diagnosis of cystic nephroma, cystic RCC, and tubulocystic carcinoma in our case.

Although immunohistochemistry is not helpful in making the diagnosis of MCRCC, it shows reactivity with MUC-1, epithelial membrane antigen (EMA), PAX-2, and carbonic anhydrase IX. In our case, EMA showed strong cytoplasmic immunoreactivity.[5]

Deletions in chromosome 3p and mutations in von Hippel–Lindau genes support that it is a type clear cell RCC.[5]

Majority of the reported cases of MCRCC were confined to the kidney and did not produce lymph nodes or systemic metastasis, suggesting a tumor of low-grade malignant potential.[1],[2],[5] Walsh et al.[6] in 2010 reported the first case of MCRCC metastasizing to intra-aortocaval lymph node.

Although surgical resection is curative, this report creates the dilemma in the management of this tumor. Clinicians have to decide whether adjuvant chemotherapy should be used in these tumors.

There is a need for more reports on large series of cases of MCRCC with follow-up studies. In our case, the patient is free from the disease for 2 years postoperatively.


  Conclusion Top


Diagnostic histopathological features of MCRCC include multiple cysts lined by fibrous septa which are lined by one or more layers of neoplastic epithelial cells having abundant clear cytoplasm and hyperchromatic nuclei with inconspicuous nuclei (Fuhrman Grade 1). The absence of necrosis and expansile tumor nodules helps in differentiating it from low-grade conventional cystic clear cell RCC. Although surgical resection is curative, lymph node metastasis is known to occur. Careful long-term follow-up is essential in these patients for better prognosis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Nassir A, Jollimore J, Gupta R, Bell D, Norman R. Multilocular cystic renal cell carcinoma: A series of 12 cases and review of the literature. Urology 2002;60:421-7.  Back to cited text no. 1
[PUBMED]    
2.
Bloom TL, Gray Sears CL, Williams TR, Linfesty RL, Amling CL. Multilocular cystic renal cell carcinoma with osseous metaplasia in a 25-year-old woman. Urology 2003;61:462.  Back to cited text no. 2
[PUBMED]    
3.
Corica FA, Iczkowski KA, Cheng L, Zincke H, Blute ML, Wendel A, et al. Cystic renal cell carcinoma is cured by resection: A study of 24 cases with long-term followup. J Urol 1999;161:408-11.  Back to cited text no. 3
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4.
Gobbo S, Eble JN, Grignon DJ, Martignoni G, MacLennan GT, Shah RB, et al. Clear cell papillary renal cell carcinoma: A distinct histopathologic and molecular genetic entity. Am J Surg Pathol 2008;32:1239-45.  Back to cited text no. 4
[PUBMED]    
5.
Suzigan S, López-Beltrán A, Montironi R, Drut R, Romero A, Hayashi T, et al. Multilocular cystic renal cell carcinoma: A report of 45 cases of a kidney tumor of low malignant potential. Am J Clin Pathol 2006;125:217-22.  Back to cited text no. 5
    
6.
Wahal SP, Mardi K. Multilocular cystic renal cell carcinoma: A rare entity with review of literature. J Lab Physicians 2014;6:50-2.  Back to cited text no. 6
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